Page 450 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )

434 PART 6 ■ Neoplastic Disorders

Prognostic Factors re rr nge ent s tr nsloc tions n eletions n nu eri-

1. Histologic l type, tu or size, nu ber o involve ly ph c l bnor lities with respect to structur l re rr nge ents.

no es n extr no l sites, presence or bsence o sys- An lysis o DNA sequences, loc te t the chro oso l

te ic sy pto s, erythrocyte se i en tion r te (ESR), bre kpoint o sever l o the recurring tr nsloc tions in leu-

l ctic ehy rogen se (LDH), lbu in, β-2- icroglobulin, ke i s or ly pho s, h s reve le th t the genes loc te

n lk line phosph t se, he oglobin, clinic l st te, n t these sites re protooncogenes. As result o the genetic

presence or bsence o involve ent o bone rrow, ut tion in uce by the chro oso l re rr nge ent, the

inguin l ly ph no es, or both re prognostic in ic tors. unction o the gene is ltere , thereby converting the gene

Ch r cteristics o the p tient inclu e ge, gen er, n the to n oncogene.

presence or bsence o ly phocytopeni .

2. Other biologic l e tures re i port nt prognostic ctors NOTE: This is a good time to complete Review Questions

in Ho gkin ise se n inclu e the seru level o soluble related to the preceding content.

CD30 ( n ntigen expresse by Ree -Sternberg cells), the

nu ber o ctiv te cytotoxic cells, n the presence or

bsence o CD15 ( n ntigen expresse by Ree -Sternberg

cells in so e v ri nt o Ho gkin ise se). PLASMA CELL DYSCRASIAS

Ger in l center, B-cell–like l rge cell ly pho h s the

best prognosis with 60% 5-ye r p tient surviv l r te er Multiple myeloma (MM) is clon l pl s cell neopl s

nthr cycline-b se che other py. It exhibite two onco- ssoci te with bnor l protein pro uction. Pl s cell

genic events not seen in the other subgroups: t(14;18) tr ns- isor ers rel te to MM re:

loc tion o the BCL2 gene n plif c tion o the c-rel locus ■ S ol ering MM (SMM)

on chro oso e 2p. P tients in the type 3 subgroup h ■ IgM onoclon l g op thy o un eter ine signif -

surviv l r te o 39%; those in the ctiv te B-cell–like sub- c nce (IgM-MGUS)

group h the poorest prognosis, with surviv l r te o 35%. ■ Non-IgM onoclon l g op thy o un eter ine sig-

nif c nce (Non-IgM-MGUS)
Treatm ent ■ Light ch in onoclon l g op thy o un eter ine

T e outco e o tre t ent or v nce Ho gkin ly pho- signif c nce

s h s i prove r tic lly over the p st two ec es. ■ Solit ry pl s cyto

Cure r tes o ore th n 70% re now possible with hybri ■ Solit ry pl s cyto with ini l rrow involve ent

regi en o echloreth ine, vincristine, proc rb zine, MM evolves ro clinic lly silent pre lign nt st ge,

pre nisone, oxorubicin, bleo ycin, n vinbl stine onoclon l g op thy o un eter ine signif c nce

(MOPP-ABV); regi en o oxorubicin, bleo ycin, vin- (MGUS). An inter e i te con ition between the lign nt

bl stine, n c rb zine (ABVD); or regi en o bleo y- MM n pre lign nt (MGUS) is SMM with uch higher

cin, etoposi e, oxorubicin, cyclophosph i e, vincristine, risk r te o progression to MM co p re to MGUS.

proc rb zine, n pre nisone (BEACOPP). R i tion ther- Plasma cell leukemia is n incre se nu ber o pl s

py h s been suggeste s benef ci l only or p tients who cells in the peripher l bloo n shoul be consi ere

h ve chieve p rti l re ission er che other py. or o ultiple yelo n not sep r te entity.

Te st n r tre t ent or p tients with i use LBCL is

cyclophosph i e, oxorubicin, vincristine, n pre nisone Epidemiology

(CHOP). Rituxi b, chi eric IgG1 onoclon l ntibo y

g inst the CD20 B-cell ntigen, h s ther peutic ctivity Multiple yelo ccounts or pproxi tely 1% o ll

in i use LBCL. T e ition o rituxi b to the CHOP types o lign nt ise ses n bout 10% o he tologic l

regi en incre ses the co plete response r te n prolongs lign ncies. MGUS is present in ore th n 3% o the pop-

event- ree n over ll surviv l in el erly p tients with i - ul tion bove the ge o 50 ye rs n progresses to yelo

use LBCLs co p re with CHOP lone without clinic lly or rel te lign ncy t r te o 1% per ye r.

signif c nt incre se in toxicity. T e onset o this isor er is between the ges o 40 n

70 ye rs, with pe k inci ence in the seventh ec e o li e.

Characteristics of Other Forms Myelo is not oun in chil ren n r rely in ults who

re less th n 30 ye rs o ge.
In NHL, Ree -Sternberg cells re bsent. T e inf ltr ting Pl s cell yelo is ore co on in en th n

cells y be o one type or y h ve ixe cell popu- wo en (1.4:1). It occurs twice s requently in A ric n

l tion o ly phocytes, histiocytes, eosinophils, n so e A eric ns s in whites.

pl s cells.

Cytogenetic Analysis Etiology

T e chro oso l no lies th t h ve been observe In ultiple yelo , typic lly, the bone rrow is involve ,

in he tologic l lign nt ise se inclu e structur l but the isor er y involve other tissues s well. T e